Exceptional stroke-like episodes in a patient with type I autosomal angioedema

摘要

We report on a man with type I hereditary angio-edema (HAE) who presented with repeated transient neurologic deficits, totally regressive after a specific C1-inhibitor (C1 INH) injection. Hereditary angioedema, first described in 1888, is an autosomal dominant disease, affecting up to 1/50,000 persons, defined in type I as a C1 INH deficiency. It is clinically characterized by recurrent edematous crisis at various body sites: mainly relapsing skin swellings, abdominal pain attacks, and life-threatening upper airway obstructions. Neurologic manifestations are exceptionally reported in this affection.